Search

J Neurol Neurosurg Psychiatry 2003;74:1403-1406 Objective: To identify the presence of vestibulo-ocular arreflexia in patients with Machado-Joseph disease (MJD), which can easily be diagnosed at the bedside. Methods: Seven patients [...]

Objective: Stimulation of the peripheral visual field has been previously reported as beneficial for cognitive performance in ADHD. This study assesses the safety and efficacy of a novel intervention involving peripheral visual stimuli in managing attention deficit hyperactivity disorder (ADHD)., Methods: One hundred and eight adults, 18-40 years old, with ADHD, were enrolled in a two-month open-label study. The intervention (i.e., Neuro-glasses) consisted of standard eyeglasses with personalized peripheral visual stimuli embedded on the lenses. Participants were assessed at baseline and at the end of the study with self-report measures of ADHD symptoms (the Adult ADHD Self-Report Scale; ASRS), and executive functions (The Behavior Rating Inventory of Executive Function Adult Version; BRIEF-A). A computerized test of continuous performance (The Conners' Continuous Performance Test-3; CPT-3) was tested at baseline with standard eyeglasses and at the end of study using Neuro-glasses. The Clinical Global Impression-Improvement scale (CGI-I) was assessed at the intervention endpoint. Safety was monitored by documentation of adverse events., Results: The efficacy analysis included 97 participants. Significant improvements were demonstrated in self-reported measures of inattentive symptoms (ASRS inattentive index; p  = 0.037) and metacognitive functions concerning self-management and performance monitoring (BRIEF-A; p  = 0.029). A continuous-performance test (CPT-3) indicated significant improvement in detectability (d'; p  = 0.027) and reduced commission errors ( p  = 0.004), suggesting that the Neuro-glasses have positive effects on response inhibition. Sixty-two percent of the participants met the response criteria assessed by a clinician (CGI-I). No major adverse events were reported., Conclusion: Neuro-glasses may offer a safe and effective approach to managing adult ADHD. Results encourage future controlled efficacy studies to confirm current findings in adults and possibly children with ADHD. Clinical trial registration : https://www.clinicaltrials.gov/, Identifier NCT05777785., Competing Interests: YR, CG, DG, NM-K, and CB-M are employees of VIZO-Specs Ltd. ZE and GV are consultants for VIZO-Specs Ltd. The author(s) declare financial support was received for the research, authorship, and/or publication of this article. This study received funding from VIZO-Specs Ltd. The funder had the following involvement with the study: study design, data collection, analysis, interpretation of data, the writing of this article, and the decision to submit it for publication., (Copyright © 2023 Richter, Gordon, Vainstein, Bublil-Mor, Geisinger, Meital-Kfir and Elyoseph.)

Objective: To evaluate the efficacy and safety of concurrent non-invasive stimulation of occipital and trigeminal nerves in acute treatment of migraine with or without aura., Background: Non-invasive neuromodulation devices stimulating a single peripheral nerve or anatomic distribution are routinely used by patients with migraine refractory to the first-line drugs or those who opt out of pharmaceutical treatment. Concurrent occipital and trigeminal stimulation was described in an invasive setting, and its safety cost outweighed its efficacy gain. This study evaluated the efficacy and safety of an external concurrent occipital and trigeminal device in acute treatment of migraine., Design and Methods: This was a randomized, sham-controlled, double-blind, multi-center trial. Patients 18 years of age or older who met the International Classification of Headache Disorders (2018) diagnostic criteria for migraine with or without aura, reported 1-6 migraine attacks per month, and other headaches no more than 6 days per month were enrolled. Of 131 intention-to-treat participants (67 and 64 in the active and sham groups, respectively), 109 (50 and 59 in the active and sham groups, respectively) treated at least one migraine episode. Reduction of migraine headache (pain relief) 2 h after treatment initiation was the primary efficacy endpoint. Pain relief at 1 h, and pain freedom and relief in most bothersome symptom at 2 h after treatment initiation were the secondary endpoints. Freedom from most bothersome symptom at 2 h and sustained pain freedom 24 h after treatment initiation were among the exploratory endpoints., Results: Sixty percent of participants (30/50) in the active arm reported pain relief at 2 h after initiation of the first eligible treatment (primary outcome) compared to 37% (22/59) in the control arm (difference, 23%; 95% confidence interval [CI], 2%-41%; p = 0.018). Pain freedom at 2 h without rescue medication was reported by 46% (23/50) of participants in the active arm and by 12% (7/59) of participants in the sham arm (p < 0.001). Pain freedom 2 h after the treatment and, subsequently, at 24 h, was reported by 4.25 times more participants in the active arm (36%; 18/50) than in the sham arm (8%; 5/59). The 28% difference was statistically significant (95% CI, 1%-43%; p < 0.001). A 4.25-fold difference was also observed comparing the proportion of participants free from pain and most bothersome symptom 2 h after the stimulation (47% [17/36] and 11% [5/45] in the active and sham arms, respectively; 95% CI, 14%-54%; p < 0.001). Adverse events were not serious or severe. All study-related events resolved without treatment., Conclusion: External concurrent occipital and trigeminal neurostimulation is a well-tolerated, safe, and effective migraine treatment that provided a fast and durable relief and freedom from migraine pain and associated symptoms in a randomized setting. The observed safety and performance suggest external concurrent occipital and trigeminal neurostimulation is a viable alternative to the currently available acute migraine treatments., Trial Registration: clinicaltrials.gov identifier NCT03631550., (© 2022 American Headache Society.)

Background: More efficient screening methods are needed to improve the ability to identify and follow genetic cohorts in Parkinson's disease (PD)., Objective: To explore the use of the electronic medical records (EMRs) to identify participants with PD., Methods: Using an algorithm previously developed in collaboration with Maccabi Healthcare Services (MHS), approximately 5,200 participants with PD were identified, more than 3,200 were screened, and 837 participants were enrolled and genotyped for leucine-rich repeat kinase 2 (LRRK2) and beta-glucocerebrosidase (GBA) variants. Questionnaires were completed to ascertain Ashkenazi Jewish (AJ) ancestry and family history of PD., Results: Among 837 participants with PD, 82% were 65 years and older and 72% had a family history of AJ ancestry. Among those with AJ ancestry, 15.6% reported having relatives with PD. The frequency of observed mutations for LRRK2 and GBA genes combined was approximately 15.4%. The frequency of observed LRRK2 mutation was 6.1% overall and 7.2% from those with AJ ancestry; and for GBA mutation was 9.3% overall and 11.2% from those with AJ ancestry., Conclusion: Although the frequency of observed mutations in this study was lower than anticipated, mutation carriers were enriched among those with a family history of AJ ancestry increasing nearly 2-3-fold, from 3% -7% (LRRK2) and 4% -11% (GBA). The identification (and selection) of PD patients through EMRs prior to genotyping is a viable approach, to establish a genetically defined cohort of patients with PD for clinical research.

Our research team previously developed an accelerometry-based device, which can be worn on the waist during daily life activities and detects the occurrence of dyskinesia in patients with Parkinson's disease. The goal of this study was to analyze the magnitude of correlation between the numeric output of the device algorithm and the results of the Unified Dyskinesia Rating Scale (UDysRS), administered by a physician. In this study, 13 Parkinson's patients, who were symptomatic with dyskinesias, were monitored with the device at home, for an average period of 30 minutes, while performing normal daily life activities. Each patient's activity was simultaneously video-recorded. A physician was in charge of reviewing the recorded videos and determining the severity of dyskinesia through the UDysRS for every patient. The sensor device yielded only one value for dyskinesia severity, which was calculated by averaging the recorded device readings. Correlation between the results of physician's assessment and the sensor output was analyzed with the Spearman's correlation coefficient. The correlation coefficient between the sensor output and UDysRS result was 0.70 (CI 95%: 0.33-0.88; p = 0.01). Since the sensor was located on the waist, the correlation between the sensor output and the results of the trunk and legs scale sub-items was calculated: 0.91 (CI 95% 0.76-0.97: p < 0.001). The conclusion is that the magnitude of dyskinesia, as measured by the tested device, presented good correlation with that observed by a physician.

The treatment of Parkinson's disease (PD) with levodopa is very effective. However, over time, motor complications (MCs) appear, restricting the patient from leading a normal life. One of the most disabling MCs is ON-OFF fluctuations. Gathering accurate information about the clinical status of the patient is essential for planning treatment and assessing its effect. Systems such as the REMPARK system, capable of accurately and reliably monitoring ON-OFF fluctuations, are of great interest., Objective: To analyze the ability of the REMPARK System to detect ON-OFF fluctuations., Methods: Forty-one patients with moderate to severe idiopathic PD were recruited according to the UK Parkinson's Disease Society Brain Bank criteria. Patients with motor fluctuations, freezing of gait and/or dyskinesia and who were able to walk unassisted in the OFF phase, were included in the study. Patients wore the REMPARK System for 3days and completed a diary of their motor state once every hour., Results: The record obtained by the REMPARK System, compared with patient-completed diaries, demonstrated 97% sensitivity in detecting OFF states and 88% specificity (i.e., accuracy in detecting ON states)., Conclusion: The REMPARK System detects an accurate evaluation of ON-OFF fluctuations in PD; this technology paves the way for an optimisation of the symptomatic control of PD motor symptoms as well as an accurate assessment of medication efficacy., (Copyright © 2017 Elsevier B.V. All rights reserved.)

[This corrects the article on p. 431 in vol. 8, PMID: 28919877.].

Background: Our group earlier developed a small monitoring device, which uses accelerometer measurements to accurately detect motor fluctuations in patients with Parkinson's (On and Off state) based on an algorithm that characterizes gait through the frequency content of strides. To further validate the algorithm, we studied the correlation of its outputs with the motor section of the Unified Parkinson's Disease Rating Scale part-III (UPDRS-III)., Method: Seventy-five patients suffering from Parkinson's disease were asked to walk both in the Off and the On state while wearing the inertial sensor on the waist. Additionally, all patients were administered the motor section of the UPDRS in both motor phases. Tests were conducted at the patient's home. Convergence between the algorithm and the scale was evaluated by using the Spearman's correlation coefficient., Results: Correlation with the UPDRS-III was moderate (rho -0.56; p  < 0.001). Correlation between the algorithm outputs and the gait item in the UPDRS-III was good (rho -0.73; p  < 0.001). The factorial analysis of the UPDRS-III has repeatedly shown that several of its items can be clustered under the so-called Factor 1: "axial function, balance, and gait." The correlation between the algorithm outputs and this factor of the UPDRS-III was -0.67 ( p  < 0.01)., Conclusion: The correlation achieved by the algorithm with the UPDRS-III scale suggests that this algorithm might be a useful tool for monitoring patients with Parkinson's disease and motor fluctuations.

Among Parkinson's disease (PD) symptoms, freezing of gait (FoG) is one of the most debilitating. To assess FoG, current clinical practice mostly employs repeated evaluations over weeks and months based on questionnaires, which may not accurately map the severity of this symptom. The use of a non-invasive system to monitor the activities of daily living (ADL) and the PD symptoms experienced by patients throughout the day could provide a more accurate and objective evaluation of FoG in order to better understand the evolution of the disease and allow for a more informed decision-making process in making adjustments to the patient's treatment plan. This paper presents a new algorithm to detect FoG with a machine learning approach based on Support Vector Machines (SVM) and a single tri-axial accelerometer worn at the waist. The method is evaluated through the acceleration signals in an outpatient setting gathered from 21 PD patients at their home and evaluated under two different conditions: first, a generic model is tested by using a leave-one-out approach and, second, a personalised model that also uses part of the dataset from each patient. Results show a significant improvement in the accuracy of the personalised model compared to the generic model, showing enhancement in the specificity and sensitivity geometric mean (GM) of 7.2%. Furthermore, the SVM approach adopted has been compared to the most comprehensive FoG detection method currently in use (referred to as MBFA in this paper). Results of our novel generic method provide an enhancement of 11.2% in the GM compared to the MBFA generic model and, in the case of the personalised model, a 10% of improvement with respect to the MBFA personalised model. Thus, our results show that a machine learning approach can be used to monitor FoG during the daily life of PD patients and, furthermore, personalised models for FoG detection can be used to improve monitoring accuracy.

A 68-year-old man presented with significant, asymptomatic, unilateral eyelash poliosis, not associated with any ophthalmic or systemic conditions. He reported that this started about 40 years ago with a few lashes in the central portion of his eyelid and that adjacent eyelashes subsequently became involved over the next 4 to 5 days. He had normal ophthalmologic and neurologic examinations with no other relevant medical history reported. The case might be an unusual presentation of a halo nevus. To the best of the authors' knowledge, this is the first such case reported regarding the eyelashes. The case and relevant literature are presented.

Freezing of gait (FOG) is a common motor symptom of Parkinson's disease (PD), which presents itself as an inability to initiate or continue gait. This paper presents a method to monitor FOG episodes based only on acceleration measurements obtained from a waist-worn device. Three approximations of this method are tested. Initially, FOG is directly detected by a support vector machine (SVM). Then, classifier's outputs are aggregated over time to determine a confidence value, which is used for the final classification of freezing (i.e., second and third approach). All variations are trained with signals of 15 patients and evaluated with signals from another 5 patients. Using a linear SVM kernel, the third approach provides 98.7% accuracy and a geometric mean of 96.1%. Moreover, it is investigated whether frequency features are enough to reliably detect FOG. Results show that these features allow the method to detect FOG with accuracies above 90% and that frequency features enable a reliable monitoring of FOG by using simply a waist sensor.

The "man-in-the-barrel" syndrome is a descriptive term that was used in the past for patients with complete flaccid paralysis of the upper extremities caused by symmetric "watershed" brain infarction after cardiovascular arrest or cardiac surgery. However, it is now evident that this syndrome can occur in acute and chronic diseases of the upper and lower motor neuron. We describe a 48-year-old woman with rapid and severe progressive weakness and wasting of both upper extremities mimicking the "man-in-the-barrel" syndrome. The neurologic and electrophysiological findings were compatible with anterior horn cell disease affecting the cervical spinal cord segments. There were only minimal signs suggesting the presence of myelopathy. After the initial rapid onset, progression during the 7 years of follow up was minimal. An extensive etiologic workup disclosed infection with HTLV-1.

Aim: To explore the epileptogenic potential of a newly introduced police car flash light device (930 Heliobe Lightbar)., Methods: A 930 Heliobe Lightbar was installed in the EEG laboratory. Thirty patients with known epilepsy, 30 subjects with chronic headache who were otherwise healthy and 15 healthy volunteers were examined., Results: All the subjects signed an informed consent and underwent an EEG during which photostimulation was performed with the standard stroboscope and later with the Police lightbar. In all 75 examined the lightbar did not induce clinical or electrographic seizures. In a single patient with epilepsy the lightbar enhanced epileptiform activity induced by standard photic stimulation., Conclusion: In this study the new Police lightbar was found to be non-epileptogenic.

There is only scant information on sleep characteristics and long-term follow-up in patients with Kleine-Levin syndrome (KLS). This study describes the clinical course, results of polysomnography and long-term follow-up in a relatively large group of patients with KLS. During the years 1982-97, we encountered 34 patients (26 males and eight females) with KLS. We were able to obtain the original polysomnographs from 28 males and four females. In 25 patients, data regarding their present state of health were obtained. Fourteen agreed to be present at a detailed interview and examination while 11 gave the information by phone. The mean age at onset was 15.8 +/- 2.8 years and the mean diagnostic delay, 3.8 +/- 4.2 years. The mean duration of a single hypersomnolent attack was 11.5 +/- 6.6 days. The main abnormal findings extracted out of 35 polysomnographs obtained from 32 patients during and/or in-between attacks included: decreased sleep efficiency, and frequent awakenings from sleep stage 2. All 25 patients reported present perfect health, with no evidence of behavioral or endocrine dysfunction. In adolescents with periodic hypersomnia, the diagnosis of KLS should be explored. Sleep recordings during a hypersomnolent period will often show frequent awakenings from sleep stage 2. The long-term prognosis is excellent.

During July-August 1998, we encountered 4 patients who suffered from syringomyelia. The diagnosis in each case could not be reached on clinical grounds only, since the characteristic thermoanalgesia dissociation was absent in all patients. In each case the spinal MRI showed the typical intraspinal cystic lesion, which finally established the diagnosis and led to appropriate treatment.

Aim: To explore the epileptogenic potential of a newly introduced police car flash light device (930 Heliobe Lightbar)., Methods: A 930 Heliobe Lightbar was installed in the EEG laboratory. Thirty patients with known epilepsy, 30 otherwise healthy subjects with chronic headache, and 15 healthy volunteers were examined. All the subjects signed an informed consent and underwent an EEG during which photostimulation was performed first with the standard stroboscope, and later with the Police lightbar., Results: In all 75 examinees the lightbar did not induce clinical or electrographic seizures. In a single patient with epilepsy, the lightbar enhanced epileptiform activity induced by standard photic stimulation., Conclusion: The new Police light bar was found to be non-epileptogenic.

The new police car flashing-light device (930 Heliobe Lightbar) has recently been implicated as potentially epileptogenic. We exposed 30 epileptic patients, 30 nonepileptic patients who suffered from headache and 15 normal volunteers to this light source. All had routine EEGs with standard intermittent photic stimulation, followed by 3-minute stimulation with the Lightbar. In none were either seizures or EEG changes induced. In 1 epileptic spike-and-wave activity induced by standard photic stimulation was enhanced with the Lightbar. We could not confirm that the Lightbar is epileptogenic.